Two cases of sporadic adult-onset neuronal intranuclear inclusion disease preceded by urinary disturbance for many years

Journal of the Neurological Sciences
Masataka NakamuraTakayuki Kondo

Abstract

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease defined by the presence of eosinophilic hyaline intranuclear inclusions. The initial and main clinical feature of adult-onset NIID is predominantly dementia. We present herein 2 cases of sporadic adult-onset NIID with longstanding urinary disturbance prior to development of other neurological symptoms. Case 1: A 71-year-old woman was admitted after she lost consciousness while bathing. She presented slowly progressive bladder dysfunction starting at the age of 40. Recently, she complained of recurrent light-headedness on standing. Her neurological findings showed miosis, muscle weakness, rigidity, hyporeflexia, sensory disturbance, cerebellar ataxia, and orthostatic hypotension. Case 2: A 68-year-old man was admitted because of episodes of transient loss of consciousness. Ten years earlier, he had developed urinary dysfunction. His neurological findings revealed cognitive dysfunction, cerebellar ataxia, and hyporeflexia. Both patients had leukoencephalopathy and motor-sensory neuropathy. In both cases, diffusion-weighted imaging showed high-intensity signals in the corticomedurally junction; and skin biopsy samples revealed ubiquitin-positive intranuc...Continue Reading

Citations

Jul 4, 2019·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Xiaoyun LiuLin Lu
Apr 7, 2020·Annals of Clinical and Translational Neurology·Pu FangDaojun Hong
Jan 4, 2020·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Hafiz Khuram RazaHao Chen
Jun 15, 2020·Journal of Neurology·Huiting LiangDaojun Hong
Nov 4, 2020·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Fen WangJianping Jia
Feb 19, 2021·Journal of Neural Transmission·Xi Lu, Daojun Hong
Aug 14, 2021·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Gao-Jia ZhangZhi-Jun Zhang
Dec 11, 2021·The American Journal of Dermatopathology·Jakob M T MoranMai P Hoang

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